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Coexistence of IgA deficiency with sarcoidosis has been reported occasionally. Enteropathy occurs exceptionally in this association. We report the case of a 45 year-old woman experiencing disseminated sarcoidosis which involved mediastinum, chest, lymph nodes, liver, bone marrow, and skin. Immunoglobulin deficiency involving essentially IgA was found 15 years later. Digestive investigations showed gastroduodenal specific localizations associated with lymphoid nodular hyperplasia without villi atrophy.
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